Uncontrolled proliferation of granulocytes
In the case of hypereosinophilic syndrome , eosinophilic granulocytes , a subgroup of leukocytes , play an important role. The leukocytes or white blood cells are responsible for the immune system. The granulocytes form in the bone marrow from myeloid stem cells. In the course of the clinical picture of hypereosinophilic syndrome, the eosinophilic granulocytes multiply uncontrollably in the blood over a period of more than 6 months – this leads to dysfunction and failure of the immune system.
Disturbance in the formation of eosinophilic granulocytes in the blood
HES related to leukocytes
Hypereosinophilic syndrome is an extremely rare blood disorder about which little scientific knowledge is available. The number of people affected is based solely on estimates, which settle at a few hundred people. Men between the ages of 20 and 50 are more likely to be affected by HES than women.
Physicians in the aetiological field are puzzling about the cause and factor for the onset of hypereosinophilic syndrome, but they identify five variants. These are:
- myeloproliferatives HES
- HES overlap syndrome (eg, hypereosinophilic dermatitis, eosinophilic pneumonia)
- familial HES
- lymphozytisches HES
- idiopathisches HES
The variants are the same disease occurring in different forms. For example, in HSE, the skin changes. This usually refers to the body but not the face.
The difficult diagnosis – recognizing the hypereosinophilic syndrome
A hypereosinophilic syndrome complicates the diagnosis due to multifaceted symptoms. The disease occurs concomitantly
- skin edema,
- Itching,
- increased levels of leukocytes,
- Fever,
- night sweats,
- weight loss,
- loss of appetite
on. If the skin is affected, eczema and macular skin changes occur. A hypereosinophilic syndrome does not only show its symptoms externally. The damage to the internal organs is far more dangerous. The disease mainly affects the heart, spleen, lungs, liver and kidneys as well as the gastrointestinal tract. The organ damage caused by the disease usually turns out to be irreparable and, in extreme cases, leads to death. The leading cause of death from HES disease is when the disease reaches the heart.
Doctors talk about HES when the high level of eosinophilic leukocytes in the blood persists for more than six months. One examination is not sufficient for a reliable diagnosis, since leukemia also has a high number of white blood cells. As with leukemia, scientists suspect an autoimmune disease in HES.
In the majority of cases, experts diagnose HES. For them, the result of molecular genetics is an important factor. Doctors diagnose a hypereosinophilic syndrome as part of the process of elimination. Various criteria are relevant for a diagnosis of exclusion. This includes:
- the proven involvement of organs.
- a blood eosinophilia greater than 1,500 µl for more than six months and
- no other diseases with an eosinophilic background are possible.
If these criteria and the results of molecular genetics are available, the diagnosis is certain.
Hypereosinophilic syndrome and treatment
Doctors treat HES in a similar way to leukemia, mainly prescribing chemotherapy . Cortisone and other medications are also used. The side effects of the medical measures are immense and do not allow those affected to lead a “normal” life. Science is working on a new active ingredient that has fewer side effects and thus helps patients.
Physicians refer patients with HES to the hospital for inpatient treatment. Therapy depends on the extent of HES and the extent to which the eosinophilic leukocytes affect the internal organs. If the heart is involved, the doctor treating you will consult a cardiologist. Depending on how badly the disease damaged the bone marrow, there are different possibilities. If the damage is severe, a bone marrow transplant is often unavoidable.
Hypereosinophilic syndrome – insights in brief
HES is a rare blood disorder that mainly affects men between the ages of 20 and 50. Diagnosis is extremely difficult due to the many different symptoms. The symptoms include the medical areas of dermatology and internal medicine. In the process of elimination, the results of the molecular technology point to the disease, provided that no other, similar disease can be considered. The blood count shows an abnormal increase in white blood cells. This is also the case with leukemia patients.
In HES, doctors monitor the level of leukocytes over a period of six months. Skin changes, fever and weight loss are among the HES symptoms. HES causes severe organic damage to the liver, spleen, lungs, heart and kidneys. In extreme cases, hypereosinophilic syndrome can lead to death. This is mainly the case when the eosinophilic leukocytes reach the heart.
Medicine is constantly gaining new insights into HES. In laboratories, scientists are looking for new, better tolerated drugs. Other researchers are investigating why, and under what circumstances, HES erupts. There are already medicines that offer good chances of recovery.
Medical note for our readers:
Our patient information explains medical topics in a simple and understandable way. Our information is not a substitute for a medical examination. Please see a specialist for diagnosis or treatment.
